Pectus Deformities

Deformed for Life?
Some children with pectus deformities are brave enough to live with their condition, and some are brave enough to undergo a challenging surgical procedure. Either way, there are sound answers for people with questions about pectus deformities. So a child can have a well-rounded chest, a well-rounded self image, or both.

What are Pectus Deformities?

Pectus deformities, the most common congenital chest wall ailments, refer to chests that are mildly, moderately, or severely misshapen. With pectus excavatum—called sunken chest or funnel chest—the sternum (breastbone) is depressed in a concave shape. With pectus carinatum—called pigeon chest—the sternum is protruded in a convex shape. The vast majority of pectus deformities (85%) are excavatum. Persons with excavatum often have a narrow chest from front to back.These chest deformities are found in one out of every 300 or so people. For those affected, they’re present at birth, and they occur in boys more than girls.

A pectus deformity is often noticeable at birth, and then becomes more apparent with every growth spurt during adolescence—and particularly during puberty. The chest’s shape and condition tend to stabilize when patients reach their late teens. Physical improvement or reversal does not occur on its own. Pectus deformities often result in an unpleasant appearance that, absent surgical correction, is overcome somewhat with maturity (chest hair, breast development, self acceptance).

The Mechanics of Pectus Deformities

Pectus deformities are thought to be caused by excessive growth of the costal cartilages, which connect the sternum to the ribs. This overgrowth buckles the ribs and pushes the sternum inward or outward. The effects of pectus excavatum and pectus carinatum differ from each other, although there are some similarities. Except in the most extreme cases, none of these effects are life threatening. There are rare cases of heart and lung malfunctions resulting from severe pectus deformities.

Symptoms of Pectus Deformities

The most obvious symptom of either type of pectus deformity is a misshapen chest—depressed or protruding. Other symptoms can include:

Excavatum:
- Displacement of heart into the left chest
- Experiencing fatigue sooner than normal
- Faster-than-normal breathing or heart rate when active
- Constrained breathing
- Compression-type pain in the lower chest
Carinatum:
- Difficulty expiring all air from the lungs
- Faster-than-normal breathing or heart rate when active
- Wheezing
- Asthma (in more severe cases)

Causes of Pectus Deformities

There is evidence of hereditary factors at work in determining candidates for pectus deformities. But what causes the costal cartilages connecting the sternum and ribs to grow beyond normal parameters and “buckle” the chest remains unknown.

Diagnosing Pectus Deformities

Because pectus deformities exist at birth, pediatricians are trained to look for them. A diagnosis of a pectus deformity is usually the result of observing an infant with a deformed chest or having difficulty breathing. The next step is an x-ray or possibly a CT (computed tomography) or MRI (magnetic resonance imaging) scan, to better understand the scope and structure of the deformity.

Treating Pectus Deformities

Pectus deformities are not life threatening, and even the most severe deformities seldom hinder normal heart and lung development. But they can interfere with one’s physical activity, and they can be rather unattractive cosmetically. Not every doctor thinks corrective surgery is needed, while the results of non-surgical treatments, for the most part, remain in question.

It can be said, however, that the vast majority of patients who opt for surgery are extremely pleased with the results. Surgery usually is recommended only after the patient has reached his or her mid-teens. That’s because there have been instances of younger patients having their chest buckle once again after surgery.

Surgical procedures include:

The Ravitch Procedure is the most common and highly successful. The sternum is disconnected from the ribs, rotated to lie flat, reconnected and then reinforced with metal rods or struts to hold its proper position during recovery. This is a major invasive procedure, which creates a sizeable scar and requires painkillers. The patient is pushed to exercise, which causes shortness of breath and forces the lungs to expand to their normal size and capacity. Only non-contact activities are allowed for four months while the bones heal. Then a second surgery is scheduled to remove the metal rods. Complications are rare and results commonly are very good, but the procedure is complex and recovery can be long.
The Nuss Procedure is a less invasive procedure developed in the 1980s to correct the more common excavatum in younger patients (age 5-15). It involves making small incisions to insert one or more metal rods behind the sternum. The bars force the sternum to its proper position and are left in place for about two years before being removed. While results have been initially encouraging, more time must pass before its long-term impact can be measured. This procedure is not recommended for older patients whose bones are thicker and more brittle, or for patients with carinatum.
The Leonard Procedure is a newer, invasive reconstructive procedure. It involves removing cartilage from the lower ribs, modifying the sternum, and rebuilding and reshaping the chest cavity using wires to reconnect and support the bones. The wires are fitted to an external plastic “Jewitt” brace, which must be worn for 6 weeks as a traction device. Like the Ravitch Procedure, this procedure is well suited for both carinatum and excavatum patients. In addition, it is being performed on patients of every age with good initial results.
Silicone implants can provide some cosmetic improvement for patients (especially older patients) with mild to moderate cases of excavatum.

Non-surgical procedures include:

Families sometimes turn to psychological counseling, because attitude can play a significant role in a patient’s well-being and adjustment to a pectus deformity. Counseling therapies can sometimes help the child develop coping strategies, establish a healthy self image, and defer or eliminate surgery as an option if that is desired. However, the impact of pectus deformities on some people exceeds the ability of counseling to overcome it.
Physiotherapies, such as posture improvement and exercises, can provide some benefit in terms of appearance and heart and lung performance. Body building exercises can worsen appearance because of larger pectoral muscles that amplify the chest deformity. But many aerobic exercises are beneficial, and any patient who slouches or has rounded shoulders would gain from certain techniques, with or without surgery.